MULTISYSTEM LANGERHANS CELL HISTIOCYTOSIS PADA

MULTISYSTEM LANGERHANS CELL HISTIOCYTOSIS PADA ANAK PEREMPUAN
USIA DUA TAHUN (LAPORAN KASUS)
MULTISYSTEM LANGERHANS CELL HISTIOCYTOSIS IN TWO YEARS OLD GIRL
(CASE REPORT)
Eky Indyanty Wuryaning Lestari1, Maimun Zulhaidah Arthamin1, Susanto Nugroho Adiwinoto2,
Budiman1
1
2
Laboratorium Patologi Klinik Fakultas Kedokteran Universitas Brawijaya
Laboratoirum Ilmu Kesehatan Anak Fakultas Kedokteran Universitas Brawijaya
Abstrak
Langerhans Cell Histiocytosis (LCH) merupakan penyakit yang jarang dengan karakteristik
proliferasi dan migrasi sel dendritik atau histiosit (sel Langerhans). Kelainan ini terutama
mengenai tulang (sistem skeletal) namun dapat juga muncul pada kulit, kelenjar tiroid, kelenjar
limfe dan risk organs involvement yaitu hepar, paru, limpa, dan sistem hematopoietik. Kelainan
ini relatif langka dan jarang sehingga diagnosis LCH sering kali terlambat atau luput. Diagnosis
pasti LCH adalah ditemukannya CD1a antigen, protein S100, atau Langerin (CD207) pada
pemeriksaan imunohistokimia atau granula Birbeck pada pemeriksaan mikroskop elektron. Pada
kasus ini seorang anak perempuan usia 2 tahun dengan keluhan mata kiri menonjol, terdapat
benjolan di belakang kepala, belakang telinga kiri dan leher sebelah kanan. Hasil pemeriksaan
fisik didapatkan proptosis okuli sinistra, massa regio occipital, belakang telinga kiri dan leher,
hepatomegali, spenomegali. Pemeriksaan laboratorium didapatkan anemia hipokrom anisositosis
mikrositik, leukositosis, trombositosis. Pemeriksaan sumsum tulang mengesankan terdapat
infiltrasi Langerhans cell histiocytosis. Pemeriksaan FNAB mengesankan Langerhans histiositosis.
Pemeriksaan foto Schuller menunjukkan lesi litik geografik tulang fronto-temporo-parietaloccipital sinistra, occipital dextra, lesi litik destruktif pada ramus mandibula dextra. Hasil CT-scan
kepala menunjukkan hasil soft tissue mass multiple. Hasil pemeriksaan imunohistokimia
didapatkan hasil positif protein S100. Pada kasus ini, pasien didiagnosis LCH atas dasar gambaran
morfologi sel Langerhans (FNAB) dan hasil positif protein S100 (imunohistokimia). Beberapa
organ yang terlibat antara lain mata, tulang craniofacial, kulit, hepar, limpa, dan sumsum tulang.
Dengan demikian pada kasus ini ditegakkan diagnosis multisystem LCH (MS-LCH).
Kata kunci : Multisystem Langerhans Cell Histiocytosis, anak usia 2 tahun, protein S100
Abstract
Langerhans Cell Histiocytosis (LCH) is a rare disease with characteristics of proliferation and
migration of dendritic cells or cell histiocytes (Langerhans cells). The disorder primarily affect the
skeletal system, but can also on the skin, thyroid gland, lymph node and risk organs involvement,
that are liver, lungs, spleen, and hematopoietic systems. The disorder is relatively rare and
seldom so that LCH diagnosis is often delayed or missed. Definitive diagnosis of the LCH is
determined of CD1a antigen, protein S100, or Langerin (CD207) on immunohistochemistry or
Birbeck granules on electron microscopy. In this case was a 2 year old girl with a protruding left
eye and a lump in the back of the head, behind the left ear and the right side of the neck. Physical
examination showed proptosis of the left occuli, the mass on occipital region, behind the left ear
and neck, hepatomegaly, spenomegaly. Laboratory tests showed hypochromic anisocytosis
anemia microcytic, leucocytosis, and thrombocytosis. Bone marrow examination showed the
infiltration of Langerhans cell histiocytes. FNAB examination showed Langerhans histiocytosis.
Schuller examination showed geographic lytic lesions on fronto-temporo-parietal-occipital
sinistra, occipital dextra, destructive lytic lesion in the mandible ramus dextra. Head CT-scan
showed multiple soft tissue mass. There was positive immunohistochemistry examination of
S100 protein. In this case, the patient was diagnosed LCH based on morphological Langerhans
cells (FNAB) and positive results of the S100 protein (immunohistochemistry). Some organs were
involved, these were eye, craniofacial bones, skin, liver, spleen, and bone marrow. Therefore, this
case enforced the diagnosis of multisystem LCH (MS-LCH).
Keywords: Multisystem Langerhans cell histiocytosis, 2 y.o children, S100 protein
Korespondensi:
Eky Indyanty W.L
Laboratorium Patologi Klinik Fakultas Kedokteran Universitas Brawijaya-RSU dr. Saiful Anwar
Malang, Jl. Jaksa Agung Suprapto Malang. Telp. (0341) 357407. Email: [email protected]